WEKO3
アイテム
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Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia
https://shinshu.repo.nii.ac.jp/records/27181
https://shinshu.repo.nii.ac.jp/records/27181068b8cd4-6034-40a8-a46c-933c519ec601
名前 / ファイル | ライセンス | アクション |
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https://soar-ir.repo.nii.ac.jp/?action=repository_action_common_download&item_id=3835&item_no=1&attribute_id=65&file_no=1
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Item type | Journal Article(1) | |||||
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公開日 | 2020-09-01 | |||||
タイトル | ||||||
タイトル | Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia | |||||
作成者(その他言語) |
Okumura, Nobuo
× Okumura, Nobuo× Terasawa, Fumiko× Takezawa, Yuka× Hirota-Kawadobora, Masako× Inaba, Tohru× Fujita, Naohisa× Saito, Masazumi× Sugano, Mitsutoshi× Honda, Takayuki |
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公開者(その他言語) | ||||||
姓名 | LIPPINCOTT, WILLIAMS & WILKINS | |||||
書誌情報 |
BLOOD COAGULATION & FIBRINOLYSIS 巻 23, 号 1, p. 87-90, 発行日 2012-01 |
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言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | albumin-binding form | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | B beta-chain | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | dysfibrinogen | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | variant dimeric fibrinogen | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
アクセス権 | ||||||
アクセス権 | metadata only access | |||||
アクセス権URI | http://purl.org/coar/access_right/c_14cb | |||||
内容記述 | ||||||
内容記述タイプ | Other | |||||
内容記述 | A heterozygous patient with dysfibrinogenemia with slight bleeding and no thrombotic complications was diagnosed with fibrinogen Kyoto VI (K-VI). To elucidate the genetic mutation(s) and characterize the variant protein, we performed the following experiments and compared with identical and similar variants that have already been reported. The proposita's PCR-amplified DNA was analyzed by sequencing and her purified plasma fibrinogen underwent SDS-PAGE followed by immunoblotting, fibrin polymerization, and scanning electron microscopic observation of fibrin clot and fibers. Sequence analyses showed that K-VI fibrinogen substituted W (TGG) for terminal codon (TAG), resulting in 12 amino acid elongation 462-473 (WSPIRRFLLFCM) in the B beta-chain. Protein analyses indicated that the presence of some albumin-binding variant fibrinogens and a dimeric molecule of variant fibrinogens reduced fibrin polymerization, with a thinner fiber and aberrant fibrin network. These results are almost the same as for the identical variant of Magdeburg, however, different from the similar variant of Osaka VI [ 12 amino acid elongation 462-473 (KSPIRRFLLFCM) in the B beta-chain] in the presence of variant forms and clot structure. We speculate the side-chain difference at 462 residues, W in K-VI, K in Osaka VI, and/or the difference in the presence of disulfide bridged forms of variant fibrinogens, led to the notable difference in the fibrin bundle network. Although a strong evolutional and structural association between B beta-chain and gamma-chain molecules is established, the corresponding recombinant 15 residue elongation variants of the fibrinogen gamma-chain showed reduced assembly and secretion. | |||||
日付 | ||||||
日付 | 2015-09-24 | |||||
日付タイプ | Created | |||||
資源タイプ | ||||||
内容記述タイプ | Other | |||||
内容記述 | Article | |||||
その他の資源識別子 | ||||||
内容記述タイプ | Other | |||||
内容記述 | BLOOD COAGULATION & FIBRINOLYSIS. 23(1):87-90 (2012) | |||||
資源識別子URI | ||||||
識別子 | http://hdl.handle.net/10091/16227 | |||||
識別子タイプ | HDL | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 0957-5235 | |||||
処理レコードID(総合目録DB) | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AA10794937 | |||||
PubMed番号 | ||||||
関連タイプ | isVersionOf | |||||
識別子タイプ | PMID | |||||
関連識別子 | http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&DB=pubmed&term=22001526 | |||||
DOI | ||||||
関連タイプ | isVersionOf | |||||
識別子タイプ | DOI | |||||
関連識別子 | http://dx.doi.org/10.1097/MBC.0b013e32834cb243 | |||||
権利 | ||||||
権利情報 | This is a non-final version of an article published in final form in BLOOD COAGULATION & FIBRINOLYSIS. 23(1):87-90 (2012) | |||||
著者版フラグ | ||||||
出版タイプ | AM | |||||
出版タイプResource | http://purl.org/coar/version/c_ab4af688f83e57aa |